How to avoid missing a systemic sclerosis sine scleroderma in a patient with pulmonary arterial hypertension


DOI: https://dx.doi.org/10.18565/therapy.2025.1.95-102

Yudkina N.N., Volkov A.V.

V.A. Nasonova Research Institute of Rheumatology, Moscow
Abstract. Connective tissue diseases (CTD) can lead to the development of pulmonary arterial hypertension (PAH) – a rare, but severe and life-threatening manifestation. The main proportion of all patients with PAH-CTD are patients with systemic sclerosis (SSc), characterized by the worst prognosis, but PAH can also be detected in systemic lupus erythematosus (SLE) and mixed connective tissue disease (MCTD), when the course of the disease is less malignant. Particularly difficult is the detection of asymptomatic, SSc sine scleroderma, in which there is no visible thickening of the skin. Wrong diagnosis can lead to untimely therapy and unfavorable outcomes.
The aim: to assess the prognosis and compare clinical and laboratory characteristics of the SSc sine scleroderma in comparison with other variants of PAH-CTD.
Material and methods. 14 patients with SSc sine scleroderma, 8 patients with SLE and 9 with PAH-associated MCTD were included in the study. CTD diagnosis was made on the basis of current diagnostic criteria, PAH was verified by cardiac catheterization. In all patients, other causes of pulmonary hypertension were excluded – heart disease, lung disease, thrombophilia.
Results. Patients with SSc sine scleroderma were significantly older (48 [35; 56] years), they were 19 times more likely to have telangiectasias, 16 times more likely to have anticentromere antibodies, and 6 times more likely to have high levels of N-terminal propeptide of B-type natriuretic hormone (NT-proBNP) and uric acid. Detection of arthritis, myositis, and antibodies to DNA increased the probability of detecting other CTDs – SLE or MCTD. Survival rate also differed: with SSc sine scleroderma, the median was 58 months, with MCTD – 68 months, and with SLE, the median follow-up was 96 months (p < 0.05).
Conclusion. Comparative analysis allowed us to identify the main predictors of SSc in patients with PAH, which can contribute to timely and early therapy and improve the prognosis of the disease.
Keywords: anticentromere antibodies, systemic scleroderma telangiectasia, systemic connective tissue diseases, pulmonary arterial hypertension, uric acid
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About the Autors

Natalya N. Yudkina, MD, PhD (Medicine), researcher at V.A. Nasonova Research Institute of Rheumatology. Address: 115522, Moscow, 34A Kashirskoe Highway.
E-mail: natudkina@mail.ru
ORCID: http://orcid.org/0000-0001-8469-8423
Alexander V. Volkov, MD, PhD (Medicine), head of the Laboratory, V.A. Nasonova Research Institute of Rheumatology. Address: 115522, Moscow, 34A Kashirskoe Highway.
E-mail: sandyvlk@yahoo.com
ORCID: http://orcid.org/0000-0003-1784-3699


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